Cyclosporin A in the treatment of acute exacerbation of idiopathic pulmonary fibrosis

Intern Med. 2010;49(2):109-15. doi: 10.2169/internalmedicine.49.2359. Epub 2010 Jan 15.

Abstract

Background: Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) is considered to be a nearly fatal condition during the clinical course of IPF, as it is unresponsive to most conventional therapies.

Subjects and methods: To evaluate the efficacy of cyclosporin A (CsA) for AE of IPF, we conducted a retrospective study on autopsied IPF cases who developed AE and were treated with corticosteroids (CS) combined with CsA. The subjects comprised 11 males with a mean age of 69.9 years. The clinical features and prognosis of the CsA-treated group was compared to a group of 11 autopsied IPF cases with a mean age of 68.7 years who developed AE and were treated with CS alone (non-CsA-treated group).

Results: CS pulse therapy followed by CS maintenance treatment were conducted in all cases of AE. Patients in the CsA-treated group received in addition a low dosage of CsA (100-150 mg). Although 7 out of 11 patients in the CsA-treated group died of AE per se, 4 patients survived the AE. Only 2 patients died during the first episode of AE. In comparison, 7 out of 11 patients in the non-CsA-treated group died during the first episode of AE. The mean survival period after the first onset of AE was 285 days in the CsA-treated group and 60 days in the non-CsA-treated group. The prognosis of the CsA-treated group therefore was significantly better than that of non-CsA-treated group after AE of IPF.

Conclusion: Administration of CsA combined with CS may be efficacious in the treatment of AE of IPF.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acute Disease
  • Aged
  • Aged, 80 and over
  • Cyclosporine / therapeutic use*
  • Disease Progression*
  • Female
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / mortality
  • Idiopathic Pulmonary Fibrosis / pathology
  • Male
  • Middle Aged
  • Retrospective Studies
  • Survival Rate / trends
  • Treatment Outcome

Substances

  • Cyclosporine